Granulomatosis with polyangiitis involving both ischemic and hemorrhagic cerebrovascular disease: A case report and literature review

Granulomatosis with polyangiitis (GPA) is a necrotizing small-vessel vasculitis associated with antineutrophilic cytoplasmic antibodies (ANCAs). GPA can have multisystem involvement; however, central nervous system (CNS) manifestations are uncommon. Here, for this first time, we report a rare case of GPA with both ischemic and hemorrhagic CNS involvement. A 67-year-old previously healthy male presented with intermittent fever, malaise, hemoptysis, skin lesions, and hearing loss. Chest computed tomography revealed multiple nodular lesions, which later enlarged and merged into consolidations. His serum ANCA titer was 1:80 with high PR3-ANCA activity. Methylprednisolone was initiated at a dose of 40 mg/day; however, only a partial response was observed. He complained dizziness and awkwardness in moving his left extremities during hospitalization, and subsequent imaging tests revealed ischemic attacks and intracranial hemorrhage in different locations. Steroid pulse therapy and rituximab were administered to induce remission. The neurological and pulmonary lesions resolved gradually. There were no signs of recurrence at 12 months post treatment. Our case highlights the involvement of CNS manifestations in GPA, and suggests potential role of rituximab in treatment of GPA patients with CNS involvement.